WOFAPS 2025 8th World Congress of Pediatric Surgery

Paolo Grassi ¹, Anna Morandi ¹, Martina Ichino ¹, Francesco Macchini ², Ernesto Leva ¹

¹ Department of pediatric surgery, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy
² Department of Pediatric Surgery, ASST Grande Ospedale Metropolitano Niguarda, Milano, Italy

Purpose: among congenital lung malformations (CLMs), extralobar pulmonary sequestrations (EPS) are generally characterized by lower surgical complexity compared to intralobar CLMs. However, atypical localizations and associated malformations require greater caution in diagnostic evaluation and surgical management. Aim of the study is to describe the localization of EPS and the associated thoracic congenital anomalies in our patients.

Method: A retrospective analysis was conducted on patients who underwent surgery for histologically confirmed EPS between 2016 and 2024 followed in our centre. The EPS location was considered atypical if different from the basal thoracic location. Preclinical and clinical data were collected and reported using descriptive statistics.

Results: During the study period, 20 patients were diagnosed with EPS, 9 (45%) having atypical locations or associated thoracic malformations. Regarding localization, 5 patients (25%) had atypical location: 2 were intradiaphragmatic, 1 intrapericardial, 1 left apical and 1 mediastinal. Four patients (20%) presented with associated malformations: 1 with an anomalous aortic vessel ending in branches of the pulmonary veins, 2 with congenital diaphragmatic hernia (CDH), and one with a diaphragmatic defect. All patients underwent sequestrectomy: 5 patients were operated on using thoracoscopic technique, 2 with laparotomic technique (those affected by CDH), and 2 via sternotomy. The mean operative time was 127 minutes. Of the 9 patients with atypical localization or associated pathologies, 8 (89%) received a prenatal diagnosis of CLM. Except for the two patients with a prenatal diagnosis of CDH, all patients underwent neonatal MRI and preoperative CT scan.

Conclusion: In our case series, 45% of EPS presented with atypical localization or associated thoracic anomalies. Especially in these cases, accurate diagnostic assessment is essential to tailor the therapeutic approach.